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DOI: 10.1148/radiol.2361031674
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Idiopathic Interstitial Pneumonias: CT Features1

David A. Lynch, MD, William D. Travis, MD, Nestor L. Müller, MD, PhD, Jeffrey R. Galvin, MD, David M. Hansell, MD, Philippe A. Grenier, MD and Talmadge E. King, Jr, MD

1 From the Department of Radiology, University of Colorado Health Sciences Center, CB A-030, 4200 E Ninth Ave, Denver, CO 80262 (D.A.L.); Departments of Pulmonary and Mediastinal Pathology (W.D.T.) and Radiology (J.R.G.), Armed Forces Institute of Pathology, Washington, DC; Department of Radiology, Vancouver General Hospital, University of British Columbia, Vancouver, British Columbia, Canada (N.L.M.); Department of Radiology, Royal Brompton Hospital, London, England (D.M.H.); Department of Radiology, Hôpital Pitié-Salpêtrière, Paris, France (P.A.G.); and Department of Medicine, San Francisco General Hospital, San Francisco, Calif (T.E.K.). Received October 15, 2003; revision requested January 12, 2004; revision received April 29; accepted July 29. Address correspondence to D.A.L. (e-mail: david.lynch{at}uchsc.edu).



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Figure 1a. Photomicrographs show UIP pattern. (a) Patchy fibrosis with lung remodeling architecture and striking subpleural distribution. Interstitial chronic inflammation is mild, with a few lymphoid aggregates (arrow). Areas of normal lung are present. There are no features of other interstitial lung disorders. Arrowhead = pleura. (Hematoxylin-eosin stain; original magnification, x4.) (b) Fibroblastic focus of loose organizing connective tissue (arrow) is seen adjacent to a dense collagenous scar. (Movat stain; original magnification, x10.)

 


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Figure 1b. Photomicrographs show UIP pattern. (a) Patchy fibrosis with lung remodeling architecture and striking subpleural distribution. Interstitial chronic inflammation is mild, with a few lymphoid aggregates (arrow). Areas of normal lung are present. There are no features of other interstitial lung disorders. Arrowhead = pleura. (Hematoxylin-eosin stain; original magnification, x4.) (b) Fibroblastic focus of loose organizing connective tissue (arrow) is seen adjacent to a dense collagenous scar. (Movat stain; original magnification, x10.)

 


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Figure 2a. Transverse CT images in a 65-year-old man with progressive shortness of breath due to UIP. (a) Left lower lobe shows peripheral ground-glass opacity and reticular patterns with traction bronchiectasis (arrows). (b) Two years later, ground-glass opacification has progressed to reticular pattern and honeycombing, with progression of traction bronchiectasis.

 


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Figure 2b. Transverse CT images in a 65-year-old man with progressive shortness of breath due to UIP. (a) Left lower lobe shows peripheral ground-glass opacity and reticular patterns with traction bronchiectasis (arrows). (b) Two years later, ground-glass opacification has progressed to reticular pattern and honeycombing, with progression of traction bronchiectasis.

 


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Figure 3a. Transverse CT images in a 78-year-old man with UIP complicated by infection with M avium-intracellulare complex. (a, b) Predominantly basal subpleural reticular pattern is present, with large left upper lobe cavity (*).

 


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Figure 3b. Transverse CT images in a 78-year-old man with UIP complicated by infection with M avium-intracellulare complex. (a, b) Predominantly basal subpleural reticular pattern is present, with large left upper lobe cavity (*).

 


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Figure 4a. Transverse thin-section CT images in a 72-year-old man with UIP complicated by large cell neuroendocrine lung cancer, which was detected incidentally on chest radiograph (not shown). (a, b) Subpleural mass in right upper lobe (arrow) is evident at two levels, with extensive, predominantly basal honeycombing.

 


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Figure 4b. Transverse thin-section CT images in a 72-year-old man with UIP complicated by large cell neuroendocrine lung cancer, which was detected incidentally on chest radiograph (not shown). (a, b) Subpleural mass in right upper lobe (arrow) is evident at two levels, with extensive, predominantly basal honeycombing.

 


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Figure 5a. Transverse CT images of accelerated UIP in a 78-year-old man with rapidly progressive shortness of breath. (a) Baseline image shows subpleural reticular pattern and honeycombing (arrows), with ground-glass opacity in remainder of the lung. (b) Five months later, after 4 weeks of progressive breathlessness, progression of honeycombing and extensive new ground-glass opacification are shown. Biopsy specimen showed organizing pneumonia pattern superimposed on background UIP. Patient responded to aggressive immunosuppressive treatment.

 


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Figure 5b. Transverse CT images of accelerated UIP in a 78-year-old man with rapidly progressive shortness of breath. (a) Baseline image shows subpleural reticular pattern and honeycombing (arrows), with ground-glass opacity in remainder of the lung. (b) Five months later, after 4 weeks of progressive breathlessness, progression of honeycombing and extensive new ground-glass opacification are shown. Biopsy specimen showed organizing pneumonia pattern superimposed on background UIP. Patient responded to aggressive immunosuppressive treatment.

 


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Figure 6a. UIP pattern in a 59-year-old man with asbestosis. (a) Transverse thin-section CT image shows predominantly peripheral honeycombing associated with reticular pattern and a lesser proportion of ground-glass opacification. (b) Transverse CT image (mediastinal window) shows bilateral noncalcified pleural plaques (arrows).

 


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Figure 6b. UIP pattern in a 59-year-old man with asbestosis. (a) Transverse thin-section CT image shows predominantly peripheral honeycombing associated with reticular pattern and a lesser proportion of ground-glass opacification. (b) Transverse CT image (mediastinal window) shows bilateral noncalcified pleural plaques (arrows).

 


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Figure 7. Photomicrograph shows NSIP with fibrosing pattern. Alveolar walls (arrows) show diffuse thickening caused by fibrosis and mild interstitial inflammation. No fibroblastic foci are present. (Hematoxylin-eosin stain; original magnification, x10.)

 


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Figure 8. Photomicrograph shows NSIP with cellular pattern. Alveolar walls (arrows) are infiltrated by a moderate chronic inflammatory infiltrate. (Hematoxylin-eosin stain; original magnification, x10.)

 


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Figure 9. Kaplan-Meier survival curves for patients with idiopathic DIP or cellular NSIP (CNSIP), fibrotic NSIP (FNSIP), and UIP. Patients with idiopathic DIP and cellular NSIP have excellent survival, those with idiopathic UIP have the worst survival, and those with idiopathic fibrosing NSIP have an intermediate survival (P < .001). (Reprinted, with permission, from reference 26.)

 


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Figure 10. Fibrotic NSIP in a 54-year-old man with shortness of breath. Transverse CT image through lower lungs shows ground-glass opacity associated with traction bronchiectasis (arrows). The left major fissure is displaced posteriorly (arrowhead), indicating lobar volume loss.

 


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Figure 11. Cellular NSIP in a 50-year-old woman with shortness of breath. Transverse CT image through lower lungs shows predominantly peribronchovascular ground-glass opacity with associated reticular pattern.

 


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Figure 12a. Reversibility of reticular pattern and traction bronchiectasis in a 58-year-old man with NSIP (mixed cellular and fibrotic pattern). (a) Initial transverse CT image shows marked basal reticular pattern with traction bronchiectasis. (b) At follow-up transverse CT performed 2 years later (without specific treatment), image obtained at a slightly lower level shows substantial resolution of reticular pattern, with residual ground-glass opacity.

 


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Figure 12b. Reversibility of reticular pattern and traction bronchiectasis in a 58-year-old man with NSIP (mixed cellular and fibrotic pattern). (a) Initial transverse CT image shows marked basal reticular pattern with traction bronchiectasis. (b) At follow-up transverse CT performed 2 years later (without specific treatment), image obtained at a slightly lower level shows substantial resolution of reticular pattern, with residual ground-glass opacity.

 


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Figure 13a. NSIP in a 62-year-old man with scleroderma. (a–c) Transverse thin-section CT images show peribronchovascular and peripheral distribution of ground-glass opacity associated with reticular pattern. Marked bilateral lower-lobe volume loss and traction bronchiectasis indicate extensive lung fibrosis. (d) Transverse thin-section CT image obtained 2 years later shows decreased ground-glass opacification but persistent reticular pattern.

 


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Figure 13b. NSIP in a 62-year-old man with scleroderma. (a–c) Transverse thin-section CT images show peribronchovascular and peripheral distribution of ground-glass opacity associated with reticular pattern. Marked bilateral lower-lobe volume loss and traction bronchiectasis indicate extensive lung fibrosis. (d) Transverse thin-section CT image obtained 2 years later shows decreased ground-glass opacification but persistent reticular pattern.

 


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Figure 13c. NSIP in a 62-year-old man with scleroderma. (a–c) Transverse thin-section CT images show peribronchovascular and peripheral distribution of ground-glass opacity associated with reticular pattern. Marked bilateral lower-lobe volume loss and traction bronchiectasis indicate extensive lung fibrosis. (d) Transverse thin-section CT image obtained 2 years later shows decreased ground-glass opacification but persistent reticular pattern.

 


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Figure 13d. NSIP in a 62-year-old man with scleroderma. (a–c) Transverse thin-section CT images show peribronchovascular and peripheral distribution of ground-glass opacity associated with reticular pattern. Marked bilateral lower-lobe volume loss and traction bronchiectasis indicate extensive lung fibrosis. (d) Transverse thin-section CT image obtained 2 years later shows decreased ground-glass opacification but persistent reticular pattern.

 


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Figure 14. NSIP pattern in a 62-year-old woman with hypersensitivity pneumonitis. Transverse thin-section CT image through left lower lung shows ground-glass opacity associated with traction bronchiectasis (arrows). Histologic analysis showed NSIP pattern, with granulomas consistent with hypersensitivity pneumonitis.

 


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Figure 15. Photomicrograph shows DIP pattern. Alveolar spaces are diffusely involved by marked alveolar macrophage accumulation, and there is mild interstitial thickening caused by fibrous connective tissue (arrows). (Hematoxylin-eosin stain; original magnification, x10.)

 


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Figure 16. Transverse CT image in a 62-year-old man with DIP who presented with shortness of breath. Predominantly basal ground-glass opacification is seen with multiple peribronchovascular cysts (arrows).

 


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Figure 17. Photomicrograph shows respiratory bronchiolitis. Faintly pigmented alveolar macrophages (arrows) fill the lumen of this respiratory bronchiole and surrounding airspaces. There is mild thickening of the respiratory bronchiole wall. (Hematoxylin-eosin stain; original magnification, x4.)

 


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Figure 18. Respiratory bronchiolitis in a 34-year-old male cigarette smoker. Transverse thin-section CT image obtained with patient in prone position shows small patch of focal ground-glass opacity (arrowhead) in posterior portion of right lung and some centrilobular nodules (arrows).

 


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Figure 19. RB-ILD in a 41-year-old man with 30 pack-year history of cigarette smoking. Transverse CT image shows widespread ground-glass opacification, with some poorly defined centrilobular nodules (arrowheads).

 


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Figure 20. Photomicrograph shows organizing pneumonia pattern. Loose plugs of connective tissue (arrows) are present in an alveolar duct and adjacent alveolar spaces. Lung architecture is preserved, and connective tissue is all the same age. (Hematoxylin-eosin stain; original magnification, x10.)

 


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Figure 21a. COP in a 75-year-old man who presented with recurrent "pneumonia."(a, b) Transverse CT images show bilateral pulmonary consolidation with subpleural and peribronchovascular predominance and right pleural effusion (arrows).

 


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Figure 21b. COP in a 75-year-old man who presented with recurrent "pneumonia."(a, b) Transverse CT images show bilateral pulmonary consolidation with subpleural and peribronchovascular predominance and right pleural effusion (arrows).

 


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Figure 22. Organizing pneumonia pattern in a 55-year-old woman with rheumatoid arthritis. Transverse CT image shows focal consolidation (arrows) in lingula, with an air bronchogram. The abnormality was not associated with symptoms of infection and resolved spontaneously.

 


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Figure 23. Photomicrograph shows diffuse alveolar damage. Lung shows diffuse alveolar wall thickening caused by proliferating connective tissue and prominent hyaline membranes (arrows). (Hematoxylin-eosin stain; original magnification, x20.)

 


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Figure 24a. AIP in a 65-year-old woman who presented with rapidly progressive shortness of breath. (a, b) Transverse CT images show extensive ground-glass opacity, with consolidation in more dependent parts of the lung and lobular areas of sparing.

 


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Figure 24b. AIP in a 65-year-old woman who presented with rapidly progressive shortness of breath. (a, b) Transverse CT images show extensive ground-glass opacity, with consolidation in more dependent parts of the lung and lobular areas of sparing.

 


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Figure 25. Acute respiratory distress syndrome secondary to trauma in a 50-year-old woman. Transverse CT image shows patchy geographic lung consolidation on the right with more diffuse consolidation in the left upper lung. A small left pneumothorax (arrow) is present.

 


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Figure 26. Photomicrograph shows LIP. Alveolar walls (arrows) are markedly infiltrated by lymphocytes and plasma cells. (Hematoxylin-eosin stain; original magnification, x20.)

 


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Figure 27. LIP caused by Sjögren syndrome in a 62-year-old woman. Transverse thin-section CT image obtained with patient prone shows diffuse ground-glass opacification and multiple lung cysts (arrows).

 


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Figure 28. American Thoracic Society and European Respiratory Society classification of IIPs: key points for the radiologist. ARDS = acute respiratory distress syndrome.

 





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